Search results for "Neuron"
showing 10 items of 2611 documents
Sporadic and Familial Variants in NF1: An Explanation of the Wide Variability in Neurocognitive Phenotype?
2020
Background: Cognitive impairment is the most common neurological manifestation in NF1 and occurs in 30-70% of NF1 cases. The onset and severity of each specific cognitive deficit varies greatly from child to child, with no apparent external causes. The wide variability of phenotype is the most complex aspect in terms of management and care. Despite multiple research, the mechanism underlying the high heterogeneity in NF1 has not yet been elucidated. While many studies have focused on the effects of specific and precise genetic mutations on the NF1 phenotype, little has been done on the impact of NF1 transmission (sporadic vs. familial cases). We used a complete neuropsychological evaluation…
Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules.
2004
AbstractPolyglutamine expansion (polyQ) in the protein huntingtin is pathogenic and responsible for the neuronal toxicity associated with Huntington's disease (HD). Although wild-type huntingtin possesses antiapoptotic properties, the relationship between the neuroprotective functions of huntingtin and pathogenesis of HD remains unclear. Here, we show that huntingtin specifically enhances vesicular transport of brain-derived neurotrophic factor (BDNF) along microtubules. Huntingtin-mediated transport involves huntingtin-associated protein-1 (HAP1) and the p150Glued subunit of dynactin, an essential component of molecular motors. BDNF transport is attenuated both in the disease context and b…
Huntingtin mediates dendritic transport of β-actin mRNA in rat neurons
2011
Transport of mRNAs to diverse neuronal locations via RNA granules serves an important function in regulating protein synthesis within restricted sub-cellular domains. We recently detected the Huntington's disease protein huntingtin (Htt) in dendritic RNA granules; however, the functional significance of this localization is not known. Here we report that Htt and the huntingtin-associated protein 1 (HAP1) are co-localized with the microtubule motor proteins, the KIF5A kinesin and dynein, during dendritic transport of β-actin mRNA. Live cell imaging demonstrated that β-actin mRNA is associated with Htt, HAP1, and dynein intermediate chain in cultured neurons. Reduction in the levels of Htt, H…
B and T lymphocytes are affected in lysosomal disorders--an immunoelectron microscopic study.
1991
Circulating lymphocytes of four patients with mucopolysaccharidoses II and IIIA, four patients with juvenile neuronal ceroid-lipofuscinosis, one patient each with glycogenosis type II, infantile neuronal ceroid-lipofuscinosis, and Gaucher disease were classified by immunoelectron microscopy as B or T lymphocytes. Disease-specific lysosomal inclusions as well as non-specific lysosomal organelles, especially Gall bodies were identified in B and T lymphocytes. These non-quantitative studies indicate that both B and T lymphocytes participate in the lysosomal storage process.
Substrate specificity overlap and interaction between Adrenoleukodystrophy protein (ALDP/ABCD1) and Adrenoleukodystrophy-related protein (ALDRP/ABCD2)
2011
X-linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder caused by mutations in the ABCD1 gene, which encodes a peroxisomal member of the ATP-binding cassette (ABC) transporter subfamily D called ALDP. ALDP is supposed to function as a homodimer allowing the entry of CoA-esters of very-long chain fatty acids (VLCFA) into the peroxisome, the unique site of their β-oxidation. ALDP deficiency can be corrected by overexpression of ALDRP, its closest homolog. However, the exact nature of the substrates transported by ALDRP and its relationships with ALDP still remain unclear. To gain insight into the function of ALDRP, we used cell models allowing the induction in a dose-dependent m…
Effects of electrical stimulation pattern on quadriceps isometric force and fatigue in individuals with spinal cord injury
2015
Introduction Variable frequency trains (VFT) or train combinations have been suggested as useful strategies to offset the rapid fatigue induced by constant frequency trains (CFT) during electrical stimulation. However, most studies have been of short duration with limited functional application in those with spinal cord injury (SCI). We therefore tested force and fatigue in response to VFT, CFT, and combined patterns in strength training-like conditions (6-s contractions). Methods Ten SCI individuals underwent either CFT or VFT patterns until target torque was no longer produced and then switched immediately to the other pattern. Results Target torque was reached more times when VFT was use…
Anticipatory postural adjustments during joint action coordination
2019
International audience; There is a current claim that humans are able to effortlessly detect others’ hidden mental state by simply observing their movements and transforming the visual input into motor knowledge to predict behaviour. Using a classical paradigm quantifying motor predictions, we tested the role of vision feedback during a reach and load-lifting task performed either alone or with the help of a partner. Wrist flexor and extensor muscle activities were recorded on the supporting hand. Early muscle changes preventing limb instabilities when participants performed the task by themselves revealed the contribution of the visual input in postural anticipation. When the partner perfo…
Biocontrol of drosophila suzukii by two fatty acids
2020
International audience; Olfaction is a crucial sense for insects to detect food, seek partners or escape dangers. Volatile molecules are mainly perceived through insects' antenna. Odors bind to specific olfactory receptors located in sensilla. Olfactory sensory neurons then synapse on corresponding specific glomeruli of the antennal lobe, the primary olfaction center. This olfactory information is processed in higher centers to trigger a behavioral response. Understanding how odors can influence insect behavior is fundamental in order to protect cultures from pests. In this work, 2 volatile fatty acids are shown to modify significantly Drosophila suzukii courtship and copulation at increasi…
Valoración de las neuronas NADPH-d en la corteza visual : estudio experimental en rata Wístar
2013
Las células NADPH-diaforasa (nicotinamida adenin dinucleotido fosfatasa reducida), son células que están directamente relacionadas con la enzima óxido nítrico sintetasa (ONS), productora del óxido nítrico (ON) neuronal. Se ha podido comprobar que la NADPH-diaforasa y la ONS se han localizado en las mismas poblaciones neuronales y que su actividad es paralela a la producción de ON y GMPc. (1). De manera que, si localizamos las células NADPH-diaforasa positivas, podremos localizar indirectamente a aquéllas que producen el ON. En cuanto a su actividad enzimática, NADPH es una enzima del grupo de las deshidrogenasas, es decir, tienen la capacidad de captar el hidrógeno de una sustancia y transf…
Neuronal structural plasticity in the Medial Prefrontal Cortex: regulation by Dopamine and PSA-NCAM
2011
Although there is a decline in brain plasticity across lifespan, neurons in certain areas of the adult brain retain the ability to undergo synaptic, dendritic and spine remodeling in response to different stimuli. This neuronal structural plasticity seems to be the basis for many cognitive processes and it is crucial for adaptive responses to aversive experiences and recovery from brain damage and disease. Among the numerous candidate molecules that have been identified for mediating this neuronal remodeling, cell adhesion molecules and, specially, the neural cell adhesion molecule (NCAM), are of particular interest. The addition of polysialic acid (PSA) to the NCAM is critical for the stru…